<i>Background:</i> Recent studies suggest an increased <i>Helicobacter pylori</i> prevalence in patients with various extradigestive inflammatory diseases. Similar to <i>H. pylori</i> infection, many respiratory diseases are characterized by chronic inflammation as well as increased immune response. Recent studies have evaluated the relation between various respiratory disorders and <i>H. pylori</i> infection. The aim of this systematic review was to scrutinize the relevant literature and the mechanisms that could underlie a role for <i>H. pylori</i> infection in respiratory diseases. <i>Methods:</i> Relevant literature regarding pathophysiological mechanisms and clinical epidemiology of <i>H. pylori</i> and different respiratory diseases has been systematically identified and analyzed by two independent reviewers according to a PubMed search for English language (until week 14, April 2010). <i>Conclusions:</i> At present, there is no definite proof of a causal relationship between <i>H. pylori</i> and respiratory diseases. Both <i>H. pylori</i> and various respiratory diseases are characterized by the release of proinflammatory cytokines and attraction of granulocytes as well as B- and T-cell-mediated response, though a pathophysiological association has not been proven. Neither the role of genetic predisposition of the host nor the presence of virulence factors nor the impact of <i>H. pylori</i> eradication have been studied in detail and definitely need further evaluation.